Use of External Distractors and the Role of Imaging Prior to Mandibular Distraction in Infants With Isolated Pierre Robin Sequence and Stickler Syndrome.

IMPORTANCE: Computed tomographic (CT) scans are often obtained before mandibular distraction osteogenesis in patients with isolated Pierre Robin sequence. There is concern regarding the risk of radiation exposure from CT in children. OBJECTIVE: To evaluate whether preoperative CT is necessary for adequate airway, feeding, and aesthetic outcomes following mandibular distraction with external distraction devices in infants with isolated Pierre Robin sequence. DESIGN, SETTING, AND PARTICIPANTS: In a retrospective review of medical records, infants who underwent mandibular distraction between January 1, 1998, and September 30, 2014, at 2 tertiary children's hospitals were identified using procedure codes. Data analysis was conducted December 1, 2014, to March 31, 2015. Fifty-two patients fit the inclusion criteria of isolated Pierre Robin sequence or Stickler syndrome, of being younger than 9 months at the time of distraction, and of use of external distractors. Forty-two of these infants did not receive preoperative CT imaging. EXPOSURE: Mandibular distraction osteogenesis for isolated Pierre Robin sequence or Stickler syndrome. MAIN OUTCOME MEASURES: Number of infants who were able to avoid tracheostomy or achieve decannulation, who were able to avoid placement or achieve removal of a gastrostomy tube, and in whom there were no intraoperative complications, no open-bite deformity, no malocclusion, no asymmetry, and no postoperative complications. RESULTS: In comparison with the 10 infants who underwent preoperative CT, all 42 of the infants (100%) who did not receive preoperative CT imaging successfully avoided tracheostomy or achieved decannulation (P = .04) and 40 patients (95%) did not require placement of a gastrostomy tube or were able to undergo gastrostomy tube removal postoperatively (P < .001). There were no significant differences between the CT and non-CT groups in the other 5 outcome measures. Two patients (5%) required postoperative gastrostomy tube placement, 2 patients (5%) had minor intraoperative complications that might have been anticipated with CT, 2 patients (5%) demonstrated malocclusion, and 1 infant (2%) had asymmetry at the end of the distraction phase. CONCLUSIONS AND RELEVANCE: This series suggests that the absence of preoperative CT does not compromise functional or aesthetic outcomes in mandibular distraction with external distraction devices in infants with isolated Pierre Robin sequence or Stickler syndrome. This finding has implications for cost containment and reduction of radiation exposure to a vulnerable population. LEVEL OF EVIDENCE: 4.

Dynamic facial reanimation with orthodromic temporalis tendon transfer in children.

IMPORTANCE: To our knowledge, orthodromic temporalis tendon transfer (OTTT) for dynamic facial reanimation has not been described for use in children. OBSERVATIONS: Three pediatric patients with permanent facial paralysis underwent OTTT using our modified technique between August 30, 2010, and January 23, 2012. Outcomes were assessed by the surgeons, patients, and patient families, with the longest follow-up period being 13 months after surgery. Two patients were 4 years old at the time of surgery, and the third patient was 17 years old. All underwent upper eyelid gold weight placement and OTTT. The hospital length of stay was 1 to 2 nights. By the first postoperative visit, all patients exhibited improved symmetry at rest, creation of a melolabial crease, and voluntary movement of the oral commissure and smile production without physical therapy. CONCLUSIONS AND RELEVANCE: The OTTT for dynamic facial reanimation in children seems to be safe and effective. To our knowledge, this is the first report of the use of this procedure in pediatric patients. This procedure has an advantage over free muscle transfer procedures in achieving immediate dynamic reanimation in a single-stage surgical procedure, without significant additional donor site morbidity. This is an important procedure in our armamentarium for treating pediatric facial paralysis.

Rib reconstruction of the absent mandibular condyle in children.

OBJECTIVES: To describe pediatric costochondral graft reconstruction of the absent mandibular condyle and to report the short-term and long-term outcomes and complications associated with performing this procedure in young children. STUDY DESIGN: Case series with a retrospective chart review. SETTING: Pediatric otolaryngology clinic and tertiary children's hospital in a metropolitan area. SUBJECTS AND METHODS: All children treated for an absent mandibular condyle with a costochondral graft at Children's Hospitals and Clinics of Minnesota were identified from 2002 through 2011, and a retrospective chart review was performed. RESULTS: Ten patients aged 3 to 11 years were identified. The most common diagnosis, in 8 of 10 patients, was oculo-auriculo-vertebral syndrome. Three of the patients had a tracheostomy, of which 1 was decannulated following condylar reconstruction. Functional improvement, defined as improved symmetry, chewing, or better oral opening, was observed in 8 of 10 patients. Five patients have required no further surgeries to date, with a mean follow-up time of 3.9 years. Severe overgrowth of the graft was noted in 1 case, and partial or complete resorption of the graft was also noted in 3 cases. Overgrowth occurred after 5.7 years, whereas resorption occurred after an average of 2.5 years. CONCLUSIONS: Costochondral grafts are an excellent surgical treatment option for children with severe mandibular malformations. Short-term results show particular improvement in function and mandibular alignment. The mean follow-up time with no revision surgery was substantial and indicates that rib grafting is a good addition to the armamentarium of treatment for this patient population.

Mandibular distraction osteogenesis in infants younger than 3 months.

OBJECTIVES: To examine the long-term outcomes and complications in infants with upper airway obstruction and feeding difficulty who underwent bilateral mandibular distraction osteogenesis (MDO) within the first 3 months of life and to identify any preoperative characteristics that may predict the long-term outcome following early MDO intervention for airway obstruction. METHODS: An institutional, retrospective medical chart review was performed. Inclusion criteria were bilateral MDO performed at an age younger than 3 months, with a minimum follow-up of 3 years. A quantitative outcome measures scale was developed, and patients were scored based on long-term postoperative complications as well as airway and feeding goals. Factors such as need for an additional surgical procedure were also considered. RESULTS: Nineteen children were identified as having undergone MDO before 3 months of age and having more than 3 years of follow-up data. The mean age at distraction was 4.8 weeks (range, 5 days-12 weeks); the mean length of follow-up was 5.6 years (range, 37-122 months). Of these 19 patients, 14 had isolated Pierre Robin sequence (PRS) and 5 had syndromic PRS. All patients with isolated PRS had a good or intermediate long-term result. Infants with comorbidities such as developmental delay, seizures, or arthrogryposis had the poorest outcomes. CONCLUSIONS: Bilateral MDO is a relatively safe and effective means of treating airway obstruction and feeding difficulty in infants with PRS. The effects of this procedure, which carries a relatively low morbidity, persist through early childhood in most patients.

Airway interventions in children with Pierre Robin Sequence.

OBJECTIVE: To describe the interventions required for successful airway management in children with Pierre Robin Sequence (PRS). STUDY DESIGN: Case series. SUBJECTS AND METHODS: The records of both a cleft and craniofacial clinic and a pediatric otolaryngology clinic were searched, and all children with PRS were identified. Data concerning feeding interventions, airway interventions, and comorbid conditions were extracted. RESULTS: Seventy-four cases of PRS were identified. Thirty-eight of the 74 children required airway intervention other than prone positioning. Fourteen of these 38 were managed nonsurgically with nasopharyngeal airway and/or short-term endotracheal intubation, whereas the remaining 24 required surgical intervention. Eighteen of the 24 underwent distraction osteogenesis of the mandible, one underwent tracheostomy, and five underwent tracheostomy followed by eventual distraction. CONCLUSION: In our series, over 50 percent of children with PRS required an airway intervention. These were both nonsurgical and surgical. As otolaryngologists, we must be prepared for the challenges children with PRS may present and the interventions that may be necessary to successfully manage these difficult airways.

Mandibular distraction to relieve airway obstruction in children with cerebral palsy.

OBJECTIVE: To determine the effectiveness of distraction osteogenesis of the mandible for relief of airway obstruction in neurologically impaired children. DESIGN: Prospective pilot study. SETTING: Tertiary care children's hospital. PATIENTS: Five children with upper airway obstruction secondary to hypotonia were identified by airway endoscopy, pulse oximetry, and polysomnography. Four children were tracheotomy-dependent because of upper airway obstruction, and 1 was being considered for tracheotomy because of progressive airway obstruction. INTERVENTIONS: All patients underwent distraction osteogenesis of the mandible for relief of their airway obstruction. MAIN OUTCOME MEASURES: Treatment success was determined by endoscopy, continuous pulse oximetry, and polysomnography. RESULTS: Four of the 5 children underwent distraction osteogenesis of the mandible with successful resolution of airway obstruction or tracheotomy decannulation. One child did not show adequate improvement of upper airway obstruction and remained tracheotomy-dependent. Follow-up was 2 to 40 months. CONCLUSIONS: Children with cerebral palsy and hypotonia of the upper airway may achieve relief of their chronic airway obstruction following distraction osteogenesis of the mandible. Appropriate selection criteria must be adhered to, including demonstration of tongue base obstruction on flexible laryngoscopy.